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- Title
NELSON SYNDROME: THE IRONY OF HAVING AN EXQUISITE FEEDBACK.
- Authors
Bulgariu, Georgiana-Loredana; Brînzan, Mihai Valeriu; Pop, George-Dorin
- Abstract
Introduction: Nelson syndrome is a rare condition characterized by a range of symptoms including visual deficits, hyperpigmentation, headache, psychological manifestations and signs of pituitary dysfunction. It arises in 26% of patients who have undergone bilateral adrenalectomy for Cushing's disease treatment. Case Report: In 2019, a 51-year-old male patient underwent bilateral adrenalectomy for refractory Cushing's disease diagnosed earlier that year. Before the initial diagnosis, the patient experienced signs and symptoms for a year including a moon-like face, red abdominal striae, centripet obesity with buffalo hump, osteoalgia and myalgia. Subsequently, in February 2024, the patient exhibited severe daily headaches localized at the right hemisphere, generalized skin hyperpigmentation, and visual disturbances suggestive of Nelson syndrome. Laboratory findings revealed elevated ACTH levels (>2000 pg/mL), PRL levels (46,9 ng/mL), indicative of a pituitary adenoma with pituitary stalk compression, and low levels of TSH and fT4. The ophthalmologic assessment identified temporal hemianopia (LE) and left superior quadrantanopia (RE). An MRI displayed a sellar region mass, compressing the optic chiasm and invading the cavernous sinuses, encasing the left intracavernous ICA.In March, the patient underwent endoscopic surgery via the transsphenoidal route and received hormone replacement therapy with glucocorticoids (Hydrocortisone) and mineralocorticoids (Astonin H). Discussions: The pathophysiological hypothesis proposes that decreased negative feedback on the hypothalamus, following surgery, may lead to the overdevelopment of the ACTH-secreting tumor. Elevated MSH levels could cause generalized darkening of the skin. The upward extension of the tumor can lead to visual disturbances by compressing the optic chiasm. Thyrotropic insufficiency is caused by the compression of the pituitary stalk and of the pituitary non-tumoral tissue. This syndrome carries significant long-term risks, including potentially fatal acute complications (pituitary apoplexy, pituitary coma) as well as enduring chronic effects (neurological impairments, visual disturbances, pain, disfigurement). Based on the Knosp classification from the MRI, this case is classified as Knosp 4, impacting surgical outcomes. In this case, the surgical remission rate is 14% with a major complication rate of 18,6%. Another approach involves patients undergoing stereotactic radiosurgery, followed by medical treatment using somatostatin-analogs and dopamine agonists.Regardless of the treatment choice, patients must have a permanent replacement therapy for glucocorticoids and mineralocorticoids. Conclusions: Though a rare complication, Nelson syndrome is a complex disorder characterized by the enlargement of the pituitary gland and the overproduction of ACTH. Every patient who undergoes bilateral adrenalectomy should be checked every 3-6 months as early detection and prompt management are crucial for enhancing patient outcome.
- Subjects
ROMANIA; ENDOSCOPIC surgery; MINIMALLY invasive procedures; CONFERENCES &; conventions; SURGICAL complications; MINERALOCORTICOIDS; ADRENALECTOMY; PITUITARY tumors; CUSHING'S syndrome; ENDOSCOPY; GLUCOCORTICOIDS
- Publication
Acta Marisiensis. Seria Medica, 2024, Vol 70, p203
- ISSN
2668-7755
- Publication type
Article